PRKAG2 syndrome is an autosomal dominant disorder first mapped to the locus 7q36 in 1991, with the responsible gene identified in 2001.3,6,7 The syndrome results from mutations in PRKAG2, which encodes the γ2 regulatory subunit of adenosine monophosphate-activated protein kinase (AMPK). This evidence concerns the gene PRKAG2 and PRKAG2-related cardiomyopathy.